Bleeding disorders

Seminar presented by : Dr. Manishpala Meda
MDS IInd Year
Dept. of Pedodontics and Preventive Dentistry

 Introduction
 History
 Hemostasis
 Platelet activation
 Coagulation cascade
 Phases of coagulation
 Role of each coagulation factor
 Anti thrombolytic mechanism
 Approach to child with bleeding disorder
 Laboratory investigations
 Classification of bleeding disorders
PART I

 Bleeding disorders are usually taken to mean coagulopathies with reduced
clotting of the blood.
 Bleeding disorders characterised by abnormal platelet function or blood
vessel walls that result in increased bleeding.
 Bleeding disorders may result from faults at many different levels in the
coagulation cascade.

 1828 - Term “haemorrhaphilia” first used.
 Later shortened to “haemophilia.”
 Hemophilia is sometimes referred to as “the royal disease,” because it affected
the royal families of England, Germany, Russia and Spain in the 19th and
20th centuries. Queen Victoria of England, who ruled from 1837-1901, is
believed to have been the carrier of Hemophilia B, or factor IX deficiency.

HEMOSTASIS: The ability of the body to control the flow of blood following
vascular injury is paramount to continued survival.
 The process of blood clotting and then the subsequent dissolution of the clot,
following repair of the injured tissue.
 It is composed of 4 major events that occur in a set order following the loss of
vascular integrity:
Coagulation and Bleeding Disorders: Review and Update
Douglas A. Triplett: Clinical Chemistry 46, No. 8(B), 2000

 Vascular constriction -limits the flow of blood to the area of injury.
 Platelet aggregation –Blood platelets clump when binding to collagen that
becomes exposed following rupture of the endothelial lining of vessels.
-Blood platelets become activated and aggregate at the site of injury .
-Upon activation, platelets release ADP and TXA2 (which activate
additional platelets).
 Clot formation -To insure stability of the initially loose platelet plug, a
fibrin mesh (also called the clot) forms and entraps the plug.
 Fibrinolysis -The clot must be dissolved in order for normal blood flow to
resume following tissue repair. The dissolution of the clot occurs through
the action of plasmin

 Normally platelets do not adhere to intact vascular endothelium.
 Subsequent to the vascular injury, platelets adhere to collagen and vWF in
the subendothelial tissue and undergo a morphological change by assuming
irregular surface, forming numerous pseudopods thus drastically increasing
their surface area.

The formation of the platelet plug involves a series of steps:
 Platelet adhesion
 After vascular injury VWf acts as a bridge between endothelial collagen and
platelet surface receptors GpIb and promotes platelet adhesion.
 The platelet glycoprotein complex I (GP-Ib) is the principal receptor for vWF.
Text book of Platelet-Vessel Wall Interactions in Hemostasis and Thrombosis
Rolando E. Rumbaut and Perumal Thiagarajan.

 Platelet secretion
 After adhesion, degranulation from both types of granules takes place with the
release of various factors.
 Release of calcium occurs here. Calcium binds to the phospholipids that appear
secondary to the platelet activation and provides a surface for assembly of various
coagulation factors.
Text book of Platelet-Vessel Wall Interactions in Hemostasis and Thrombosis
Rolando E. Rumbaut and Perumal Thiagarajan.

 Platelet aggregation:
 Thromboxane A2 produced by activated platelets
provide stimulus for further platelet aggregation.
 TxA2 along with ADP enlarge this platelet aggregate
leading to the formation of the platelet plug, which
seals off vascular injury temporarily.
 ADP binding also causes a conformational change in
GpIIb/IIIa receptors presents on the platelet surface
causing deposition of fibrinogen.
 Thrombin generation also catalyses the conversion of
this fibrinogen to fibrin which adds to the stability of
the platelet plug and is now known as secondary
haemostasis.

 It is based on cell based theory of coagulation
 Intiation phase : The initiation phase is localized to the cells that express TF.
 The FVIIa/TF complex activates small amounts of FIX and FX.
 FXa associates with its cofactor, FVa, and forms a prothrombinase complex
on the surface of the TF-bearing cell.
Theories of Blood Coagulation: James P. Riddel Jr, MS, RN, CPNP Bradley E. Aouizerat, PhD Christine
Miaskowski, RN, PhDDavid P. Lillicrap, MD, FRCPC:Journal of Pediatric Oncology Nursing, Vol 24, No 3
(May-June), 2007: pp 123-131

 Amplification phase: A small amount of thrombin generated on the
TFbearing cell has several important functions.
 A major function is the activation of platelets, exposing receptors and binding
sites for activated clotting factors.
 As a result of this activation, the platelets release partially activated forms of
FV onto their surfaces.
 Thrombin activates platelets, cofactor FVA and FVIII on the platelet surface
and FXI on platelet surface.

 Propagation Phase: As large numbers of platelets are recruited to the site of
injury, the propagation phase of clot formation occurs on the surface of
activated platelets.
 First, FIXa activated during the initiation phase can now bind to FVIIIa on the
platelet surface.
Theories of Blood Coagulation: James P. Riddel Jr, MS, RN, CPNP Bradley E. Aouizerat, PhD Christine
Miaskowski, RN, PhDDavid P. Lillicrap, MD, FRCPC:Journal of Pediatric Oncology Nursing, Vol 24, No 3
(May-June), 2007: pp 123-131

Termination Phase:
 Once a fibrin platelet clot is formed over an area of injury, the clotting process
must be limited to avoid thrombotic occlusion in surrounding normal areas of
the vasculature
 Three types of natural anticoagulants regulate clotting: antithrombin (ATIII)
inhibits the activity of thrombin and other serine proteases, such as FIXa, FXa,
FXIa, and FXIIa.

 Fibrinogen (factor I): consists of three polypeptide
chains - alpha, beta and gamma.
 It is converted to fibrin (factor Ia) by thrombin
(factor IIa).
 Fibrin forms a mesh around the wound ultimately
leading to blood clot.
 The inherited disorders caused due to mutations in
fibrinogen include: Afibrinogenemia (complete lack
of fibrinogen), Hypofibrinogenemia (reduced levels
of fibrinogen) Hyperfibrinogenemia (dysfunctional
fibrinogen).
 These individuals suffer from thromboembolism.
 The gene for factor I is located on the fourth
chromosome.
Overview of the coagulation system: Sanjeev Palta, Richa singh:
Indian journal of anesthesia; vol 25 sept-0ct 2014

 Tissue factor (factor III) :
 It is also called as platelet tissue factor.
 It is found on the outside of blood vessels and is not exposed to the
bloodstream.
 It initiates the extrinsic pathway at the site of injury.
 It functions as a high-affinity receptor for factor VII.
 It acts as a cofactor in the factor VIIa-catalyzed activation of factor X to FXa.
 The gene for tissue factor is located on the first chromosome.

 Factor V :
 Is also referred to as proaccelerin or labile factor.
 It is enzymatically inactive and acts as a cofactor to the serine protease FXa,
which in the presence of calcium ions and an appropriate phospholipid (PL)
membrane surface enhances the activation of prothrombin to thrombin.
 Factor V Leiden mutation causes factor V deficiency or parahemophilia, which is
a rare bleeding disorder.
 It may also lead to myocardial infarction and deep vein thrombosis.
 The gene for factor V is located on the first chromosome (1q21-q25).

 Factor VII:
 Vitamin K-dependent serine protease.
 It initiates coagulation by activating factors IX and X simultaneously with tissue
factor in the extrinsic pathway.
 Its deficiency may lead to epitaxis, menorrhagia, hematomas, hemarthrosis,
digestive tract or cerebral haemorhages.
 The gene for factor VII is located on the thirteenth chromosome (13q34-qter).

 Factor VIII:
 Is also known as anti-hemophilic factor (AHF).
 It is a cofactor in the activation of factor X to FXa, which is catalyzed by factor
IXa in the presence of calcium and phospholipids.
 Mutations in the factor VIII gene results in Hemophilia A. It is also called
classical hemophilia, an X-linked recessive coagulation disorder.
 It is the most common type of hemophilia. Pateints suffer from clinical
manifestations in their early childhood; spontaneous and traumatic bleeds
continue throughout their life.
 The gene for factor VIII is located on the long arm of X chromosome (Xq28).

Factor IX:
 Is also known as Christmas factor.
 It is a proenzyme serine protease, which in the presence
of calcium activates factor X.
 Its deficiency cause Hemophilia B or Christmas disease.
 Although, the clinical symptoms of hemophilia A and B
are similar, hemophilia B is less severe than hemophilia
A.
 High antigen or activity levels of factor IX is associated
with an increased risk of thromboembolism.
 The gene for factor IX is located on the X chromosome
(Xq27.1-q27.2).

Factor X:
 Is also known as Stuart-Prower factor.
 In the presence of calcium and phospholipid, it functions in
both intrinsic and extrinsic pathway of blood coagulation.
 Factor X is activated to FXa by factors IX and VII. It is the
first member of the common pathway of blood coagulation.
 FXa cleaves prothrombin to thrombin.
 Its deficiency may cause bleeding diathesis and
hemorrhages.
 Patients commonly suffer from epitaxis, gastrointestinal
bleeds and hemarthrosis. Women with factor X deficiency
may be susceptible to miscarriages.
 The gene for factor X is located on the thirteenth
chromosome (13q32-qter).

Factor XI :
 Is also known as plasma thromboplastin antecedent.
 It is a serine protease zymogen which is activated to factor XIa
by factor XIIa.
 Deficiency in factor XI causes injury-related bleeding.
 The disorder is sometimes referred to as Hemophilia C.
 Individuals with severe deficiency do not show excessive
bleeding conditions and hemorrhage normally occurs after
trauma or surgery.
 Female patients may experience menorrhagia and prolonged
bleeding after childbirth.
 The gene for factor XI is located on the distal end on the
long arm of fourth chromosome (4q35).

Factor XII :
 Is a plasma protein, also known as Hageman factor.
 It is the zymogen form of factor XIIa, which
activates factor XI and prekallikrein.
 Its deficiency does not cause excessive hemorrhage
due to lack of involvement of factor XIIa in
thrombin formation.
 However, it may increase the risk of thrombosis,
due to inadequate activation of the fibrinolytic
pathway.
 The gene for factor XII is located on the tip of the
long arm of the fifth chromosome (5q33-qter).

Factor XIII or fibrin stabilizing factor:
 It is the proenzyme form of plasma transglutaminase.
 It is activated by thrombin into factor XIIIa in presence of
calcium.
 It forms ε-(γ-glutamyl)-lysyl bonds between the fibrin
chains and stabilizes the blood clot.
 Thus, it reduces the sensitivity of the clot to degradation by
proteases.
 Genetic defects in the factor XIII gene leads to lifelong
bleeding diathesis.
 Patients may also suffer from intercranial bleeding and
death.
 The gene for F13A is located on the sixth chromosome
(6p24-25). The F13B gene is located on the long arm of
first chromosome (1q32-32.1)

 Von Willebrand factor (VWF):
 Is a multimeric glycoprotein involved in hemostasis.
 It supports binding of platelets to the site of injury by forming a bridge
between collagen matrix and platelet-surface receptor complex.
 Hereditary or acquired defects of vWF lead to von Willebrand disease.
 Patients may suffer from bleeding diathesis, menorrhagia and gastrointestinal
bleeding.
 The gene for vWF is located on short arm of the twelfth chromosome

 It is therefore important that the endothelial
surface provides several proteins and receptors
that inhibit thrombin and counteract the progress
of coagulation.
 The potent thrombin inhibitor antithrombin III
binds to specific heparan sulfates of
proteoglycans in the glycocalyx that covers the
endothelial surface .
 Inadequate antithrombin III activity is
accompanied by severe thrombotic complications.
 Furthermore, a triad of endothelial receptors is
involved in the generation the important anti-
coagulant activated protein C (aPC), which can
interrupt the coagulation cascade by cleavage of
factors Va and VIIIa

The history should determine:
 The site or sites of bleeding, the severity and
duration of hemorrhage, and the age at onset.
 Was the bleeding spontaneous, or did it occur
after trauma?
 Was there a previous personal or family
history of similar problems?
 If a child or adolescent has had surgery that
affects the mucosal surfaces, such as a
tonsillectomy or major dental extractions, the
absence of bleeding usually rules out a
hereditary bleeding disorder

PHYSICAL EXAMINATION
 The P/E should focus on whether bleeding symptoms are associated primarily
with the mucous membranes or skin (mucocutaneous bleeding) or with the
muscles and joints(deep bleeding).
 The examination should determine the presence of petechiae, ecchymoses,
hematomas, hemarthroses, or mucous membrane bleeding.
 Patients with defects in platelet-blood vessel wall interaction (VWD or
platelet function defects) usually have mucocutaneous bleeding.
 Individuals with a clotting factor deficiency of factor VIII or IX (hemophilia A
or B) have symptoms of deep bleeding into muscles and joints.
 Individuals with disorders of the collagen matrix and vessel wall may have
loose joints and lax skin associated with easy bruising (Ehlers-Danlos
syndrome).

 Blood count and film
 show the number and morphology of platelets and any blood disorder such
as leukaemia or lymphoma.
 The normal range for the platelet count is 150- 400 × 109/L
 Bleeding time
 measures platelet plug formation in vivo
 normally between 3 and 10 minutes
 Prolonged bleeding times are found in patients with platelet function
defects
TEXT BOOK of medical physiology: Guyton and hall :12 th Edition

 Prolonged bleeding times are found in patients with platelet function
defects.
 Performed using blood collected into citrate, which neutralizes
calcium ions and prevents clotting.
 The prothrombin time (PT)
 The partial thromboplastin time (PTT)
 The thrombin time (TT)
 Correction tests
 Factor assays
 Special tests of coagulation

 Coagulation tests
 The prothrombin time (PT)
 Time needed for the plasma to clot in the presence of tissue
thromboplastin and calcium
 Evaluates the ability of blood to clot properly
 Normal time for clotting is 10-14s
 Prolong PT results from def of:
 Factor V
 Factor VII
 Factor X
 Prothrombin
 Fibrinogen

Seminar presented by : Dr. Manishpala Meda
MDS IInd Year
Dept. of Pedodontics and Preventive Dentistry
PART II

 Types of bleeding disorders
 Coagulation factor deficiencies
 Hemophillia A
 Hemophillia B
 Von willibrands disease
 Vitamin k deficiency
 Liver disease
 Platelet disorders/ coagulation factor deficiencies
 DIC
 Conclusion
 References


Coagulation factor deficiencies:
 Congenital
 Hemophilia A and B
 Von Willebrand’s disease
 Other factor deficiencies (rare)
 Acquired
 Liver disease
 Vitamin K deficiency, warfarin use
 Disseminated intravascular coagulation

Platelet disorders:
Quantitative disorder (thrombocytopenia)
 Immune-mediated
 Idiopathic
 Drug-induced
 Collagen vascular disease
 Sarcoidosis
 Non-immune-mediated
 Microangiopathic hemolytic anemia
 Leukaemia
 Myelofibrosis

Qualitative disorder
 Glanzmann thrombasthenia
 Liver disease
 Alcoholism

Vascular disorders
 Scurvy
 Purpura
 Hereditary hemorrhagic telangiectasia
 Cushing syndrome
 Ehlers-Danlos syndrome
Fibrinolytic defects
 Streptokinase therapy

Coagulation factor deficiencies

 Hemophilia A is due to a deficiency of clotting factor VIII or antihemophilic
factor or Hemophilia B is due to deficiency of clotting factor IX (hemophilia B).
 Inherited X-link disorder
 Prevelance 1 in 5000 in male population.
Naveen Kumar J, Anil Kumar R, Varadarajan R, Sharma N. Specialty dentistry for the hemophiliac: Is there a
protocol in place? Indian J Dent Res 2007;18:48-54

 Clinical features
 Atypical profuse bleeding at circumcision
 Bruising at neonatal vaccines
 Joints and soft tissue bleeds and excessive bleeding when they start to
be active
 Prolonged bleeding after teeth extraction
 Recurrent painful hemarthrosis
 Muscle haemoatomas
 Spontaneous haematouria
 GIT hemorrhage
 Spontaneous intracranial hemorrhage (rare)
Naveen Kumar J, Anil Kumar R, Varadarajan R, Sharma N. Specialty dentistry for the
hemophiliac: Is there a protocol in place? Indian J Dent Res 2007;18:48-54

 The clinical severity depend on the level of factor VIII:C = severity of
condition
 Severe = factor level < 1%
 Moderate = factor level 1 – 5%
 Mild = factor level > 5 %

 Severe disease – factor level < 1%
 Frequent spontaneous bleeding from early life
 Haemarthroses are common and may lead to joint deformity
 Bleeding into muscles is also common
 Moderate disease – factor level 1 – 5 %
 Post traumatic Bleeding
 Occasional apparently spontaneous episodes
 Mild disease – factor level > 5 %
 Usually with bleeding only after injury or surgery
 Diagnosis in this group is often delayed until quite late in life
Naveen Kumar J, Anil Kumar R, Varadarajan R, Sharma N. Specialty dentistry for the
hemophiliac: Is there a protocol in place? Indian J Dent Res 2007;18:48-54

 Laboratory finding – investigations
 Coagulation testing
 Prolonged activated partial thromboplastin time (APTT)
 Normal prothrombin time (PT)
 Normal bleeding time (BT)
 Factor assay (reduced level of factor VIII)

 Also known as Christmas disease
 Caused by a deficiency of factor IX
 The inheritance and clinical features are identical to hemophilia A
 Only can be distinguished by specific coagulation factor assays
 The incidence is only about 1 in 30 000 males
 Hemophilia B is treated with factor IX concentrates

 General Management
1. Factor replacement
Bleeding is treated by administration of factor VIII concentrate by intravenous infusion.
 Minor bleeding: the factor VIII:C level should be raised to 20-30%
 Severe bleeding: the factor VIII:C should be raised to at least 50%
 Major surgery: the factor VIII:C should be raised to 100% preoperatively and
maintained above 50% until healing has occurred.
The following formulas were applied for calculating the factor dose:
Dosage (units) = body weight (kg) × desired factor VIII rise (IU/dL or % of
normal) × 0.5
Dosage (units) = body weight (kg) × desired factor IX rise (IU/dL or % of
normal)x 0.5

2. Synthetic vasopressin (Desmopressin)
 An analogue of vasopressin
 Intravenous, subcutaneous or intranasal
 Produces a rise in factor VIII:C in mild hemophilia
 It avoids the complications associated with blood products
 It is ineffective in severe haemophilia

3.Tranexamic acid is a synthetic derivative of lysine, available for topical and
systemic usage. However, nausea is a common adverse effect.
4.The anti-fibrinolytic agent Epsilon amino caprioic acid (EACA) given
orally of IV is a potent inhibitor of initial clot dissolution. A regimen of 50
mg/kg body weight EACA given orally as a 25% oral rinse every six hours
for seven to ten days appears adequate as an adjunct.
Still tranexamic acid is 10 times more potent than EACA with fewer side-
effects.

Difficulties in the management of a hemophiliac dental patient include the
following:
 Dental neglect necessitating frequent extractions
 Trauma and surgery
 Factor VIII inhibitors (recombinant FVIIa is an alternative Solution)
 Hazards of anesthesia and injections
 Risk of hepatitis B and liver disease and HIV infection
 Aggravation of bleeding by drugs
 Anxiety and drug dependence
Scully C, Cawson RA. Medical problems in dentistry. 5th ed. Elsevier: London; 2005.

 The bleeding tendency can be aggravated by NSAIDs. Safer alternatives for
pain control are acetaminophen, codeine and Cox-2 inhibitors.
 Local anesthetic regional blocks, lingual infiltrations or injections into the
floor of the mouth must not be used in the absence of Factor VIII replacement
because of the risk of hemorrhage hazarding the airway and being life-
threatening.
 If FVIII replacement therapy has been given, regional LA can be used
provided the FVIII level is maintained above 30%.
 Infiltrations, intraligamentary, intraosseous or intrapulpal injections are still
safer.

 Restorative treatment can be undertaken routinely providing care is taken to
protect the mucosa. There is a risk of bleeding with the use of matrix bands or
wooden wedges. This can be controlled by local means or the application of
topical agents.
 Cotton rolls should be wetted before removal.
 High-speed vacuum aspirators and saliva ejectors can cause hematomas.
 Trauma from the saliva ejector can be minimized by resting it on a gauze
swab in the floor of the mouth.
Brewer A, Correa ME. Guidelines for dental treatment of patients with inherited bleeding disorders.
Treatment of hemophilia. 2006; 40.

 Avoiding instrumentation through the periapex is of prime importance in
endodontic therapy.
 The presence of bleeding in the canal is indicative of pulp tissue remaining
in the canal.
 Sodium hypochlorite should be used for irrigation in all cases, followed by
the use of calcium hydroxide paste to control the bleeding.
 Formaldehyde-derived substances may also be used in cases where there is
persistent bleeding or even before the pulpectomy.
Batawi HYE. Minimizing the risk of perioperative bleeding in a child with hemophilia A during dental
rehabilitation under general anesthesiia: a case report. Int J Clin Ped Dent 2013; 6(3): 217-222.

 Isolation with rubber dam provides retraction of gingiva and improves
visibility.
 It also minimizes the potential for laceration of the buccal mucosa and lips.
 Notches may be placed in buccal and lingual surfaces with a fissure bur
into which clamp prongs will fit tightly.

 Mild hemophiliacs requiring surgeries can be managed usually without factor
replacements.
 Desmopressin and tranexamic acid are primary alternatives.
 Desmopressin can be as a slow intravenous infusion over 20 min of 0.3-0.5
µg/kg, 30 to 60 min prior to the surgical procedure.
 This results in a two- to threefold rise in Factor VIII activity with a mean half-
life of 9.4h.
Borea G, Montebugnoli L, Capuzzi P, Magelli C. Tranexamic acid as a mouthwash in anticoagulant-
treated patients undergoing oral surgery. An alternative method to discontuing anticoagulant therapy. Oral
Surg Oral Med Oral Pathol 1993; 75: 29-31

 Intranasal administration as a spray of 1.5 mg per ml with each 0.1 ml pump
spray is an alternative, limiting treatment to those hemophiliac patients whose
basal factor levels are sufficiently high.
 Tranexamic acid significantly reduces blood loss and can be given topically
or systemically
Borea G, Montebugnoli L, Capuzzi P, Magelli C. Tranexamic acid as a mouthwash in anticoagulant-
treated patients undergoing oral surgery. An alternative method to discontuing anticoagulant therapy. Oral
Surg Oral Med Oral Pathol 1993; 75: 29-31

 Healthy periodontal tissue is essential to prevent bleeding and tooth loss.
 If oral hygiene is poor treatment must start as soon as possible after the patient
has had a dental examination and treatment plan formulated to prevent
additional damage to the periodontal tissues.
 In cases of severe periodontal disease, it may be necessary to carry out
supragingival scaling initially along with oral hygiene education.
 Subgingival scaling can start as soon as the inflammation has decreased. The
treatment may need to be carried out over several visits to prevent excessive
blood loss.
 In addition, chlorhexidine gluconate mouthwash can be used to control
periodontal problems.
 Antibiotics may be required to help reduce the initial inflammation.
Naveen Kumar J, Anil Kumar R, Varadarajan R, Sharma N. Specialty dentistry for the hemophiliac: Is there
a protocol in place? Indian J Dent Res 2007;18:48-54

 Dental pain can usually be controlled with a minor analgesic such as
paracetamol (acetaminophen). Aspirin should not be used due to its inhibitory
affect on platelet aggregation.
 The use of any non-steroidal anti-inflammatory drug (NSAID) must be
discussed beforehand with the patient's hematologist because of their effect on
platelet aggregation
 A buccal infiltration can be used without any factor replacement. It will
anesthetize all the upper teeth and lower anterior and premolar teeth.

 Contact the hemophilia unit and consider using additional factor concentrate.
 Inspect the site of the bleed.
 If there is any evidence of a tear in the gingiva or other obvious bleeding point
this should be treated using local measures.
 Instruct the patient to sit up and bite on a damp gauze swab for at least 10
minutes.
 Use a 10% solution of tranexamic acid or EACA to dampen the swab or as a
mouthwash if the bleeding is difficult to stop.
 Monitor the patient’s blood pressure as it may increase due to worry and pain.

 In some hemophilia centres, fibrin glue is used as a local hemostatic measure,
along with an oral antifibrinolytic agent, to achieve hemostasis and reduce the
need for clotting factor replacement therapy.
 All fibrin glue contains human or animal components, which has made a
number of physicians and patients being hesitant to use this treatment
particularly for patients who are receiving recombinant factor concentrates or
have never received blood products derived from humans.

 A 7-year and 3-month-old known mild hemophilic child who
was under the regular care of a hematologist and a
pediatrician in a private hospital in Jeddah, Saudi Arabia.
Medical History :
 The patient had no other medical disorders or history of
surgeries.
 The patient had multiple incidents of nasal bleeding that
required admission to the same hospital.

The intraoral examination revealed the following:
 Missing teeth, numbers 51 and 52 due to normal shedding
 Decayed teeth, numbers 55, 74, 46 and 85
 Badly decayed and infected teeth, numbers 54, 64 and 84
 Potential for crowding in the lower anterior segment
The treatment plan included the following:
 Composite resin restoration for teeth numbers 55, 74, 46 and 85
 Pulpotomy (ferric sulfate) and steel crowns for teeth numbers 65 and 75
 Extraction for teeth 54, 64 and 84
 Chairside ready-made space maintainers in place of teeth 54, 64 and 84
 Mesial slicing of the lower canines for temporary relief of crowding in the
lower incisor region
 Sealant applications for teeth 16, 26 and 36
 Topical fluoride application

 The child was admitted to the hospital 48 hours prior to the operation for factor VIII
replacement therapy using the following formula:
 FVIII dose (IU/kg) = (desired increase in FVIII IU/dl) ÷ 2 to maintain an FVIII
level of 70 IU/dl.
 This level was recommended by the hematologist based on the type of teeth to be
removed (primary or permanent) and the anticipated amount of trauma that might be
exerted during the dental procedures as stated by the pediatric dentist.
 The preoperative blood investigation results were taken. The child was advised and
educated to perform gentle brushing with a soft toothbrush and to use a povidone
iodine mouthwash after meals.
 The parents were instructed to stop meals for at least 6 hours prior to the time of
surgery.
 Preoperative complete blood counts showed low hemoglobin (10.6 g/dl); other
values were within the normal range according to the patient’s age group

 Oral intubation in a small child requires good intraoral
 Space management for proper accessibility and vision.
 During the course of the dental treatment, the mouth probe was inserted in a
reversed position to allow good retraction of the tube, which was
accommodated inside the probe to economize the use of the intraoral space
 Use of local anesthesia, even for patients undergoing treatment under general
anesthesia, to minimize postoperative pain and aid patient homeostasis during
surgery.
 The current case was not an exception to this routine. Nerve block injections
were avoided to lower the risk of hematoma and/or internal bleeding

Positioning the Child
 Children with hemophilia can suffer from internal bleeding of the joints and
muscles should these tissues become strained.
 Adequate support for the knees and neck is routinely provided for all children
undergoing dental care under general anesthesia, with special concern for
hemophilic children.
 Hygoformic saliva ejectors (Orsing Co.) were used to minimize possible
trauma and subsequent bleeding risk . The suction holes of these tips were
directed away from mucosal tissue.

Extractions
 Avoiding unnecessary trauma was a primary concern.
 The following were considered:
1. Elective root division was used, and each root was extracted individually .
2. Plastic instrumentation was used to remove broken down teeth instead of
root elevators to minimize the use of excessive force.

Postoperative Care
 The patient was hospitalized for 48 hours, during which he continued factor
VIII replacement as proposed by his hematologist and pediatrician.
 Oral amoxicillin and clavulanic acid postoperative antibiotics (457 mg/5 ml )
were administered every 12 hours for 7 days.
 For postoperative pain control, the preferred drug was acetaminophen 200 mg,
administered every 8 hours for 3 days.

Dental management of hemophiliac child under general anesthesia JOURNAL OF INDIAN SOCIETY
OF PEDODONTICS AND PREVENTIVE DENTISTRY | Jan - Mar 2011 | Issue 1 | Vol 29 |
A 4-year-old boy came along with the parents the exclusive child specialty
dental center with the complaints of pain in the right upper back tooth
region

 Hereditary coagulation abnormality caused by either:
 Reduced level of vWF
 Abnormality in vWF
Due to Point mutation or Major deletion
Guidance on the dental management of patients with haemophilia and congenital bleeding disorders J. A.
M. Anderson,*1 A. Brewer,1 D. Creagh,1 S. Hook,1 J. Mainwaring,1 A. McKernan,1 T. T. Yee1 and C. A.
YeungBRITISH DENTAL JOURNAL VOLUME 215 NO. 10 NOV 23 2013

Vwf is a protein plays two role in action:
 It promote adhesion of platelets to the endothelium
 It is a carrier molecule for factor VIII, protecting it from premature
destruction
So in Vwd :
 Defective platelet function
 Factor VIII:C deficiency

CLASSIFICATION OF VWD:
3 TYPES:
 TYPE I:
 Characterized by a mild reduction in VWF
 usually inherited as an autosomal dominant.
 TYPE II:
 Loss of high-molecular-weight multimers, and it too is usually inherited as an
autosomal dominant
 TYPEIII:
 Characterized by severe reduction in VWF and usually inherited as
autosomal recessive.

 Typically there is mucus membrane bleeding (epistaxis, menorrhage...)
 The severity of symptoms are variable with types
 Type 1, 2 usually mild symptoms
 Type 3 severe symptoms

 Laboratory Investigations:
 The bleeding time is prolonged
 APTT is prolonged
 Factor VIII is low
 VWF is usually low (type 1,2)
 Platelets count is normal

Treatment
 Depends on the severity of the condition.
 May be similar to that of mild haemophilia, including the use of
Desmopressin where possible.
 Factor VIII or Von Willebrand factor concentrates should be used to treat
bleeding or to cover surgery in patients who require replacement therapy.

 Factor XI deficiency
 Rare
 Seen mainly in Ashkenazi Jews
 Caused bleeding only after trauma
 Treated by factor XI
 Factor XII deficiency
 Usually cause no bleeding

 Acquired coagulation disorders
 More common than inherited disorders
 Usually multiple clotting factors
 Includes
 Vitamin K deficiency
 Liver disease
 Coagulation disorders caused by antibodies
 Massive transfusion syndrome

Vitamin K is a fat soluble vitamin
 Obtained from green vegetables and bacterial synthesis in the gut
 Important on coagulation factors II, VII, IX and X and on proteins C and S.
 Without it, these factors cannot bind calcium.
 Hemorrhagic disease of the newborn
 Biliary obstruction
 Malabsorption of vitamin K
 Vitamin K antagonist drugs

 Biliary obstruction results in malabsorption of vitamin K and therefore
decreased synthesis of factors II, VII, IX and X.
 Also there are decreased in factor V and fibrinogen.
 Dysfibrinogenemia.
 Thrombocytopenia.
 Functional abnormalities of platelets.
 Hypersplenism associated with portal hypertension.
 DIC.

 There is widespread deposition of fibrin within blood vessels with
consumption of coagulation factors and platelets occurs as a
consequence of many disorders which release procoagulant material
into the circulation or diffuse endothelial damage or generalized
platelet aggregation
Bleeding Disorders of Importance in Dental Care and Related Patient Management Anurag Gupta,
BDS; Joel B. Epstein, DMD, MSD, FRCD(C); Robert J. Cabay, MD, DDSJCDA • • February 2007,
Vol. 73, No. 1 •

• Infections
– Gram neg septicemia
– Septic abortion
• Malignancy
– Widespread mucin secreting
adeno-carcinoma
– Acute promyelocytic
leukemia
• Hypersensitivity reactions
– Anaphylaxis
– Incompatible blood
transfusion
• Obstetric complications
– Amniotic fluid embolism
– Eclampsia, retained
placenta
• Widespread tissue damage
– Following surgery/trauma
– After severe burns
• Miscellaneous
– Liver failure
– Severe burns
– Hypothermia
– Snake venoms
– Acute hypoxia
Vol. 73, No. 1 •

 DIC may triggered by the entry of procoagulant material into circulation:
 Amniotic fluid embolism
 APML
 Premature separation of placenta
 Initiated by widespread endothelial damage and collagen exposure:
 Septicemia
 Severe burns
 Widespread intravascular platelet aggregation
 Some bacteria, viruses and immune complexes may have direct effect
on platelets.
Vol. 73, No. 1 •

 Bleeding, particularly from venipuncture
 Purpura
 Generalized bleeding in GIT, oropharynx,
lungs, urogenital tract, vaginal bleeding
 Less frequently, microthrombi may cause
skin lesions, renal failure, gangrene of
fingers

 Laboratory finding
 The platelet count is low
 Fibrinogen low
 Thrombin time is prolonged
 High level of fibrin degradation products (FDP)
 PT and APTT are prolonged in acute syndromes
 Blood film
 Fragmentation of red cells
Vol. 73, No. 1 •

Treatment
 Treat underlying cause
 Supportive therapy with fresh frozen plasma (FFP) and platelets
concentrates
 Cryoprecipitate may also required
Vol. 73, No. 1 •

 If there is active bleeding, consultation with a hematologist may be
considered to assist with definitive diagnosis and, for patients who are
ultimately diagnosed with a bleeding disorder, individualized long-term
hemostatic management.
 Treatment planning is essential for good outcome and should involve liaison
between the dentist and the haemophilia centre.
 Consideration should be given to careful scheduling of invasive dental
procedures to minimise re-exposure to factor concentrate.
 Written post-procedural instructions should be provided and must include
emergency contact numbers.

Bleeding Disorders of Importance in Dental Care and Related Patient Management Anurag Gupta, BDS;
Joel B. Epstein, DMD, MSD, FRCD(C); Robert J. Cabay, MD, DDSJCDA • • February 2007, Vol. 73, No.
1 •
Dental management of hemophiliac child under general anesthesia JOURNAL OF INDIAN SOCIETY
OF PEDODONTICS AND PREVENTIVE DENTISTRY | Jan - Mar 2011 | Issue 1 | Vol 29 |

Naveen Kumar J, Anil Kumar R, Varadarajan R, Sharma N. Specialty dentistry for the hemophiliac: Is
there a protocol in place? Indian J Dent Res 2007;18:48-54
Scully C, Cawson RA. Medical problems in dentistry. 5th ed. Elsevier: London; 2005.
Overview of the coagulation system: Sanjeev Palta, Richa singh:
Indian journal of anesthesia; vol 25 sept-0ct 2014

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